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Biomedical subjects

D Sidi

Publications and source records attributed to D Sidi.

At least 19 recordsLinked to original sources

[Wolff-Parkinson-White syndrome in the child. A case report with associated atrial fibrillation].

UNLABELLED: In children, Wolff-Parkinson-White (WPW) syndrome is often revealed by reentrant tachycardia episodes. Rarely, this syndrome can be the cause of syncope or of sudden death, resulting from rapid conduction of atrial fibrillation to the ventricles through the accessory pathway. CASE REPORT: The authors report the case of a child, who had a WPW syndrome diagnosed after birth because of a supraventricular tachycardia and who was later asymptomatic. At ten years of age, he was admitted because of palpitations due to atrial fibrillation and rapid conduction through the accessory pathway. CONCLUSION: In a child with WPW syndrome, tachycardia with wide and irregular QRS complexes should evoke the involvement of atrial fibrillation, with rapid conduction to the ventricle. Drugs blocking conduction through the atrio-ventricular node are contra-indicated and the condition is an indication for radiofrequency ablation.

Atrial Fibrillation↗

[Percutaneous pulmonary valve replacement: towards a modification of the prosthesis].

Percutaneous pulmonary valve replacement has recently been introduced as an alternative to surgery, pushing back the frontiers of interventional catheterisation. The authors report their experience in this field and focus on the complications of the method. From September 2000 to November 2002, 14 patients with a failing artificial pulmonary outflow tract were included in this protocol of clinical research. The authors attempted pulmonary implantation of a bovine jugular vein valve fitted in a vascular stent under general anaesthesia. The implantation was possible in all patients. Three had dynamic obstruction due to the implanted valve. This was due to infiltration of blood between the valve and the stent. The problem was resolved by: implanting a second valve in one patient: redialating the valve stent in two other patients. However, the last patient had to be reoperated 7 weeks after the implantation because of recurrence of the obstruction. One valve had to be explanted one year after implantation because of severe infectious endocarditis after unprotected dental treatment. Two patients experienced fracture of the stent but without valve dysfunction. Despite these complications, the clinical state of all patients improved during follow-up. The authors conclude that non-surgical pulmonary valve replacement is feasible, thereby increasing the therapeutic arsenal of interventional catheterisation. Simple modifications should result in fewer problems of dynamic obstruction and stent fracture, and lead to proposing this technique for native pulmonary outflow tracts.

Adolescent↗

[Experience of implantation of prefitted Palmaz Corinthian stents].

We report our recent experience with the Palmaz Corinthian stent in children. Since 1/10/01 to 1/11/02 we implanted 18 Palmaz Corinthian stents in 12 children. Median age and weight of patients were 5.6 years (range 0.25-13) and 17.9 kg (range 3-36), respectively. Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 7), complex transposition of the great arteries (n = 2) and others (n = 3). Indications to stent implantation were: pulmonary branches stenosis (n = 9), stenosis of the ductus arteriosus (n = 1), stenosis of systemic veins (n = 2). All stents were pre-mounted and co-axially advanced over a standard guidewire across the stenotic site, using a 6F short introducer. Stent deployment was performed by hand inflation of the balloon. Final angiography confirmed correct positioning and ruled out complications. Median fluoroscopy time was 18 minutes (range 12-56). All stents reached their nominal diameter; 2 were post-dilated to 125 and 150%. No procedural complications occurred. Despite the impossibility of distal injection, stent positioning was always correct. In conclusion, due to the availability of pre-mounted stents, and the need of short 6F sheaths, the use of the Palmaz Corinthian stent in children is relatively simple. Primary results are generally good in terms of adequate delivery and absence of complications. However, the impossibility of distal injection makes more difficult the choice of the deployment site. Due to the limited possibility of over-dilation, the use of this stent has to be limited to the stenosis of peripheral pulmonary branches or to lesions that can be surgically treated during further surgical procedures.

Adolescent↗

[Early complications of stenting in children and young adults with congenital heart disease].

We report the early complications of stent implantation. Since 1/1/97 to 1/12/02, we implanted 91 stents in 76 patients. Median age and weight were 15 years (range 0.3-43) and 49 kg (range 3-96). Primary diagnosis were: pulmonary atresia with ventricular septal defect (n = 17), tetralogy of Fallot (n = 13), aortic coarctation (n = 15), transposition of the great arteries (n = 3), truncus arteriosus (n = 6), univentricular heart (n = 7), other anomalies (n = 28). Indications to stent implantation were: stenosis of the pulmonary outflow and/or pulmonary branches (n = 44), aortic recoarctation (n = 14), native aortic coarctation (n = 1), systemic venous channels obstruction (n = 14), other (n = 3). The stents were: Palmaz (n = 57), non premounted CP (n = 8), premounted CP (n = 3), Palmaz Corinthian (n = 19), self-expanding (n = 4). Procedural complications occurred in 24 patients (31%) and included: 11 balloon bursting, 8 stent slipping, 1 stent migration, 4 wall dissections. Five patients (21%) needed urgent surgery. Sixty three percent of complications occurred during pulmonary outflow or pulmonary branches stenting, 25% during stenting of the aortic arch, 12% during other procedures. All but 2 complications were observed with non premounted stents. In conclusion, the use of not premounted stents, although widely diffuse, has a not negligible incidence of complications, leading to longer procedures, to the use of a large variety of materials and needing more than occasionally a surgical solution. A larger use of premounted stents, when possible, is recommended.

Adolescent↗

[Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiopathy].

The late occurrence of complete atrio-ventricular block (CAVB) after cardiac surgery is rare but potentially responsible for cases of late sudden death. We searched for factors allowing prediction of this complication, retrospectively reviewing the case notes of 11 patients in hospital with complete AVB, 2 months to 10 years after correction of a cardiac malformation. All had a normal pre-operative ECG. The diagnosis had been made based on symptoms in 8 patients: syncope or collapse (4 cases) symptoms on effort (3 cases) or fatigue (1 case). In the others the diagnosis had been made on ECG. The block was infra-His in 5 patients who had electrophysiology. ECG analysis showed that all the patients had CAVB immediately post-operatively lasting 3-14 days. After restoration of conduction the ECGs showed the following anomalies compared to the pre-operative ECGs: long PR (1 case), long PR + right bundle branch block (2 cases), long PR + left axis deviation (1 case), RBBB + left deviation or rotation of the QRS axis (3 cases), long PR + RBBB + left axis deviation (4 cases). All of these patients had been fitted with a cardiac stimulator. In conclusion, the children who had CAVB immediately post-operatively lasting more than 48 hours and who then had an ECG showing different QRS compared to the pre-operative QRS and/or long PR had a risk of late complete AVB. These patients should have electrophysiology and a stimulator must be implanted in those who have an infra-His block.

Bundle of His↗

[Cardiac retransplantation in childhood. Questions and answers].

Cardiac transplantation in childhood remains a long-term palliative procedure and it is probable that an increasing number of patients undergoing this procedure in childhood will require a new transplant one day. Coronary artery disease of the transplanted heart is the main indication. The results (in terms of survival and morbidity) in the literature and in the authors' experience are encouraging and justify the indication for retransplantation in cases of severe coronary disease of the transplanted heart. The indication is not as clear-cut in transplantation performed early or as an emergency for primary graft dysfunction, which carries a high mortality. Renal failure and allo-immunisation are not contra-indications to this procedure. Occasionally, procedures of coronary revascularisation provide time to wait for a new transplant. In the absence of more effective preventive or curative treatment of coronary disease of the transplant, the good results of retransplantation justify the procedure for this indication and reinscription on the waiting list for cardiac transplantation.

Adolescent↗

Assessment of muscular ventricular septal defect closure by transcatheter or surgical approach: a three-dimensional echocardiographic study.

AIMS: Previous classification of muscular ventricular septal defects (VSDs) visualized on two-dimensional echocardiography relied on artificial divisions of the septum. New visualization of the ventricular septum integrating the third dimension would facilitate communication between cardiologists and surgeons. The objectives of this study were (1) to assess in patients with muscular ventricular septal defects the accuracy of left ventricular three-dimensional echocardiographic reconstructions in demonstrating the position, the size and the tissue rims of the defects; (2) to compare findings by three-dimensional echocardiography with those obtained by surgical and transcatheter approaches. METHODS AND RESULTS: Twenty-six patients, aged from one month to 40 years, with muscular ventricular septal defects underwent three-dimensional echocardiographic study. From the left ventricular three-dimensional echocardiographic reconstructions, the localization, the maximal diameter and the tissue rim of the defect were analysed and compared with surgical or transcatheter findings. Optimal three-dimensional echocardiographic reconstructions were obtained in 22 patients. Nineteen had a single muscular ventricular septal defect and three had multiple muscular ventricular septal defects. The muscular ventricular septal defect localizations were the inlet septum in three, the outlet septum in three, the mid-muscular septum in 14 and the apex in eighth. In 10 patients who underwent surgical closure, the correlation between three-dimensional echocardiography and surgery for muscular ventricular septal defect maximal diameter was y=0 x 95 x +0.13 (r=0.98; P<0.001). The agreement between three-dimensional echocardiographic and intraoperative findings on muscular ventricular septal defect localization were complete. In five patients who underwent transcatheter closure, the mean difference between three-dimensional echocardiographic maximal diameter and stretched diameter was 1 x 8+/-0 x 5 mm. CONCLUSION: The three-dimensional echocardiographic left ventricular views provide a new and easily communicated visualization of various muscular ventricular septal defects. Such new imaging should contribute to the surgical and transcatheter treatments of muscular ventricular septal defects.

Adolescent↗

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.

BACKGROUND: Friedreich's ataxia encodes a protein of unknown function, frataxin. The loss of frataxin is caused by a large GAA trinucleotide expansion in the first intron of the gene, resulting in deficiency of a Krebs cycle enzyme, aconitase, and of three mitochondrial respiratory chain complexes (I-III). This causes oxidative stress. Idebenone, a short chain quinone acting as an antioxidant, has been shown to protect heart muscle against oxidative stress in some patients. OBJECTIVE: To assess the efficiency of idebenone on cardiac hypertrophy in Friedreich's ataxia. DESIGN: Prospective, open trial. SETTING: Tertiary care centre. METHODS: Idebenone (5 mg/kg/day) was given orally to 38 patients with Friedreich's ataxia aged 4-22 years (20 males, 18 females). Cardiac ultrasound indices were recorded before and after idebenone treatment. RESULTS: After six months, cardiac ultrasound indicated a reduction in left ventricular mass of more than 20% in about half the patients (p < 0.001). The shortening fraction was initially reduced in six of the 38 patients (by between 11-26%) and it improved in five of these. In one patient, the shortening fraction only responded to 10 mg/kg/day of idebenone. No correlation was found between responsiveness to idebenone and age, sex, initial ultrasound indices, or the number of GAA repeats in the frataxin gene. CONCLUSIONS: Idebenone is effective at controlling cardiac hypertrophy in Friedreich's ataxia. As the drug has no serious side effects, there is a good case for giving it continuously in a dose of 5-10 mg/kg/day in patients with Friedreich's ataxia at the onset of hypertrophic cardiomyopathy.

Adolescent↗

[3D-transthoracic echocardiography: a selection method prior to percutaneous closure of atrial septal defects].

Prior to putting in place a percutaneous device, the assessment of the atrial septal defect anatomy is mandatory. The 3D transthoracic echocardiography is a non-invasive method bringing an imaging of the septal defect surface and its borders. Fifty-two patients ageing from 3 to 16 years old had a rotational 3D transthoracic echocardiography. Thirty-six (69%) were selected for a percutaneous closure (borders > 7 mm and a septal surface/atrial septal defect ratio > 2). Thirty-two of these selected patients (89%) benefited from the Amplatz prosthesis implantation with success. The maximal 3D diameter of the septal defect was 20 + 4 mm (14-30) compared to the mean size of prosthesis at 22 mm (18-30). Four of the 36 patients (aneurismal septum or a borderline septal surface/atrial septal defect ratio) were secondarily oriented to surgeons. Sixteen patients (31%) were selected directly to a surgical closure. The lack of borders or septal surface were confirmed by the surgical view. The 3D transthoracic echocardiography allows to define new criteria for the selection of patients prior to a percutaneous closure of atrial septal defects. This prospective study demonstrates that 9 out of 10 patients who had the 3D criteria had a percutaneous closure of the septal defect.

Adolescent↗

[Marfan syndrome in the newborn and infants less than 4 months: a series of 9 patients].

We report a series of 9 children with neonatal Marfan syndrome. The diagnosis was made on a striking facial dysmorphia associated with arachnodactyly type skeletal anomalies. They all had cardiovascular anomalies: aortic dilatation 9 times, and mitral and/or tricuspid valve prolapse 8 times. Three children underwent ascending aorta replacement with the Yacoub technique. There was one operative death in a young infant and the two survivors required further surgery for gross mitral insufficiency and an aneurysm of the horizontal aorta. Three children died before the age of 1 year from cardiac insufficiency linked to massive leaks of all the valves. The three youngest children in the series did not undergo any procedure and were followed up for a slowly growing dilatation of the initial aorta. The prognosis of neonatal Marfan syndrome is weighted with a heavy mortality. The timing of surgical intervention in case of excessive dilatation of the aortic root is difficult to determine. It depends not only on the aortic diameter but also on the aortic valve function and the existence of other mitral or tricuspid lesions. Surgical treatment for lesions of the initial aorta and the atrioventricular valves improves the short-term prognosis but the risk of re-intervention is higher.

Aortic Diseases↗

[Percutaneous closure of a paravalvular mitral regurgitation with Amplatzer and coil prostheses].

The authors report a case of percutaneous closure of paravalvular mitral regurgitation in a 9 year old child. This patient had complete atrioventricular canal with levo-isomerism completely repaired with replacement of the left atrioventricular valve by a mechanical prosthesis. One year after an episode of infectious endocarditis, the patient was readmitted because of jaundice due to haemolysis attributed to paravalvular mitral regurgitation. Transoesophageal echocardiography demonstrated a crescent-shaped dehiscence of the prosthesis opposite the left atrial appendage associated with a small paraseptal regurgitation. First of all, a 6 mm Amplatzer Septal Occluder and a 5 x 5 coil were introduced by retrograde catheterisation to treat the regurgitation due to the valve dehiscence. This procedure was completed 3 months later by the implantation of a second 8 mm Amplatzer prosthesis for persistent haemolysis. Closure of the paraseptal regurgitation resulted in a reduction of the patient's transfusional requirements.

Child↗

[Percutaneous closure of an interatrial communication via the internal jugular route using an Amplatzer prosthesis].

Closure of interatrial communications by interventional catheterisation is recommended and effective. The usual approach is via the femoral vein. However, difficulty in positioning the probe and the guide in a stable fashion across the septal defect occurred during closure of the interatrial communication by the percutaneous route in a 71 year old female with severe scoliosis. This deformity most certainly contributed to the failure of the femoral approach. The decision for closure by the internal jugular route was taken and allowed closure of the interatrial communication to be achieved. The internal jugular route can be an alternative for closure of interatrial communications with difficult access.

Aged↗

[Followup of adults with a Fontan-type cavopulmonary derivation].

The Fontan procedure is a technique used in single ventricle syndromes and initiates a circulation which bypasses the right ventricle. It was described by Fontan in 1971. Many variations of this operation have been described and classified under the term of total cavopulmonary derivation. These operations result on long-term functional benefit. However, the 15 year survival rate is only 55 to 80%. This procedure (often carried out in childhood) is considered to be palliative rather than curative. The mortality is due to long-term complications, the frequency of which increases with the length of follow-up of the patient. The causes are: the Fontan circulation itself (arrhythmias, thrombosis, inferior caval stasis (hepatic, exsudative enteropathy), recurrence of cyanosis): the underlying cardiac disease (ventricular failure, atrioventricular valvular regurgitation); other complications (endocarditis and extracardiac, in particular orthopedic problems). Any of these complications may be an indication for cardiac transplantation, a high risk procedure because of previous multiple surgical interventions.

Adult↗

[Geographic origin of endomyocardial fibrosis treated at the central hospital of Maputo (Mozambique) between 1987 and 1999].

Endomyocardial Fibrosis (EMF) is a restrictive myocardiopathy of unknown aetiology, which is specific to tropical areas. In order to document the geographical distribution of this disease in Southern Mozambique, we reviewed the medical records of patients treated at Maputo Central Hospital between 1987 and 1999. Analysis was restricted to cases originating from the 3 provinces of Southern Mozambique. Cumulative incidences were computed for the period of 1987-1999, by age group, sex and residential district at disease onset. The geographical distribution of the disease in the 34 districts (administrative subdivisions) of the study area was mapped. Between 1987 and 1999, 118 cases of EMF originating from Maputo, Gaza and Inhambane provinces were diagnosed at Maputo Central Hospital. Seventy seven patients (65%) were living in Inhambane Province at the time of disease onset. Cumulative incidence for this province was 6.9/100,000 over the study period of 1987-1999. Comparatively, cumulative incidences were respectively 1.7/100,000 for Maputo and 1.0/100,000 for Gaza Province (chi 2 = 64.3, p < 10(-5)). All the patients were native from 22 out of the 34 districts of these 3 provinces. Affected districts were all located in the coastal area and higher cumulative incidence was computed for Inharrime district (18.3/100,000). This study confirmed that endomyocardial fibrosis is endemic in some areas of South Mozambique, but did not allow to measure the true magnitude of the disease which is probably grossly underestimated. The geographical distribution of EMF in South Mozambique reinforce the environmental hypothesis of aetiologic disease.

Adolescent↗

Presence of increased stiffness of the common carotid artery and endothelial dysfunction in severely obese children: a prospective study.

BACKGROUND: Epidemiological studies suggest that obesity-induced atherosclerosis may start in childhood, but this process has never been demonstrated. We looked for arterial changes and investigated their relation to cardiovascular risk factors in obese children. METHODS: Non-invasive ultrasonographic measurements were made in 48 severely obese children and 27 controls to investigate arterial mechanics and endothelial function. Plasma lipid concentrations, indices of insulin resistance, and body composition were assessed in the obese children. FINDINGS: The obese children had significantly lower arterial compliance than the healthy controls (median 0.132 [0.022-0.273] vs 0.143 [0.112-0.237] mm(2).mm Hg; p=0.02) and lower distensibility (0.60 [0.10-1.00] vs 0.70 [0.50-1.10] mm Hg(-1).10(-2); p=0.0001). Conversely, the obese children had higher values than the controls for wall stress (3.36 [2.00-5.01] vs 2.65 [2.13-3.54] mm Hg.10(2); p=0.0001) and incremental elastic modulus (1.68 [0.72-10.8] vs 0.96 [0.64-1.47]; p=0.0001). Endothelium-dependent and independent function were also lower in the obese than in the control children. An android fat distribution was positively correlated with indices of insulin resistance and plasma triglyceride concentrations and was negatively correlated with plasma HDL-cholesterol concentration and arterial compliance. Endothelial dysfunction was correlated with low plasma apolipoprotein A-I and with insulin resistance indices. INTERPRETATION: Severe obesity in children is associated with arterial wall stiffness and endothelial dysfunction. Low plasma apolipoprotein A-I, insulin resistance, and android fat distribution may be the main risk factors for these arterial changes, which are of considerable concern as possible early events in the genesis of atheroma.

Adolescent↗